We present an interesting case of a 2½-year-old child with Tetrology of Fallot with a large intracardiac mass in the left ventricle presenting with fever and bilateral stroke, that resolving with antitubercular therapy alone.

OBJECTIVE: To retrospectively analyze the experience of one-stage surgical correction for children with congenital heart diseases and tracheal stenosis and further to clear the principle of treatment for these patients. METHODS: From January 2006 to June 2013, 48 patients with congenital heart diseases and tracheal stenosis underwent surgical correction. There were 36 male and 12 female patients. The mean age at operation was (23 ± 27)months (range: 3-72 months) and the mean weight was (12 ± 8) kg (range: 3...

A 12-years-old male child presented with polydactyly and syndactyly of hands, hypospadias, AND undescended testes. He was a known case of Tetrology of Fallot. One important differential in our case was Bardet-Biedl syndrome, but it was ruled out due to lack of evidence of central obesity, mental retardation and retinitis pigmentosa. At this time, there is no molecular testing available to distinguish these two syndromes.

The case of a 16 years old female with uncorrected Tetrology of Fallot, who operated for intracranial lesion in parieto frontal area with midline shift is presented. She had right ventricular hypertrophy, clubbing, central and peripheral cyanosis. Patient was anaesthetized keeping all measures required to avoid haemodynamic swings, tachycardia, desaturation, acidosis and dehydration. Pre-operative antibiotic cover was given to prevent bacterial endocarditis. Neurosurgeon, Paediatric cardiologist, Anaesthesiologist and Intensivist were involved in the preoperative planning and management of the patient...

Sinus of Valsalva aneurysm (SVA) is a rare disease that is mostly congenital in origin. The rupture of a SVA may occur and can eventually lead to death unless definite surgical treatment occurs. In this article, a ruptured and percutaneously treated SVA in a subject who underwent two consecutive corrective open cardiac surgeries due to a congenital heart disease was examined. An 18-year-old male was referred because of the complaint of dyspnea and fatigue. He underwent a complete repair of tetrology of Fallot before and a redo-repair of residual VSD three years before...

OBJECTIVE: To assess the prevalence, pattern and outcome of congenital heart disease (CHD) among the babies born in Bhabha Atomic Research Centre (BARC) Hospital, Mumbai and to compare the pattern of CHD with the study done in the same hospital in year 1999. METHODS: A prospective, longitudinal study was conducted from January 2006 through December 2011. Babies presenting with murmur were closely followed up till the diagnosis and further to assess the outcome. 2-D Echocardiography was used as a diagnostic tool...

Congenital heart disease (CHD) is one of the most common birth defects in humans. Mutations in cardiac transcription factor genes, such as GATA4, NKX2-5 and TBX5 genes, have been associated to a small portion of familial and isolated CHD cases. NKX2-5, a highly conserved homeobox gene, is expressed in the developing heart. During embryonic development, NKX2-5 plays pivotal roles in specifying cardiac progenitors, cardiac morphogenesis, cardiomyocyte differentiation and conduction system development. Numerous mutations in NKX2-5 gene have been reported in CHD patients, including atrial septal defect, ventricular septal defect (VSD) and tetrology of Fallot...

Percutaneous closure of perimembranous and muscular ventricular septal defects is becoming well established worldwide. We present the case of a 15-year-old girl with postoperative tetrology of Fallot and a residual ventricular septal defect that was closed with Amplatzer device complicated by early device migration and severe tricuspid valve obstruction.

Tetrology of Fallot (TOF) is the most commonly encountered congenital cardiac lesion in pregnancy. Although there are controversies regarding safe anesthetic technique for parturient with TOF, we use low-dose sequential combined-spinal epidural anesthesia in such a case posted for Cesarean section and found that low dose (0.5 ml of 0.5%) intrathecal bupivacaine and fentanyl with sequential epidural bupivacaine supplementation was adequate for the performance of an uncomplicated Cesarean section with minimal side effects and good fetal outcome...

To assess the maternal and perinatal outcome in pregnant women with cardiac disease, a prospective study was carried out among 51 pregnant women with cardiac disease between January 2006 and August 2008 at a tertiary care centre at BJ Medical College, Ahmedabad, Gujarat. Rheumatic cardiac disease (68.62%) with mitral valve involvement (88.57%) was the commonest cardiac disease. In congenital cardiac disease (21.57%) group septal defect was found as the predominant form. Cardiac surgeries were performed in 11 women before pregnancy out of which 7 had balloon valvotomies, 3 had valve replacements and one had corrective tetrology of Fallot surgery...

Seckel syndrome, also known as Harper's Syndrome and Virchow-Seckel-Syndrome, was first described by Seckel in 1960, and is a rare (<1:10,000 live births) autosomal recessive trait. The syndrome (OMIM 210600) is a form of primordial dwarfism, characterized by severe intrauterine growth restriction, postnatal dwarfism, severe microcephaly with variable mental retardation, facial anomalies and skeletal abnormalities. Cardiac anomalies have been described in only five case. We report a male newborn with typical findings of Seckel sydrome associated with Tetrology of Fallot...

OBJECTIVE: To analyze the outcome of continuous versus interrupted closure technique of ventricular septal defect (VSD) closure in Tetrology of Fallot with reference to postoperative residual VSD after total correction. METHODS: A randomised control study was conducted between January 2008 to December 2008 at The Department of Cardiac Surgery, National Institute of Cardiovascular Diseases (NICVD), Karachi. The results of total correction (T.C) of VSD in patients with Tetralogy of Fallot, with emphasis on the suturing technique and eventually on the occurrence of residual ventricular septal defect(VSD) were analyzed...

BACKGROUND: Flow mapping by cardiovascular magnetic resonance has become the gold standard for non-invasively defining cardiac output (CO), shunt flow and regurgitation. Previous reports have highlighted the presence of inherent errors in flow mapping that are improved with the use of a stationary phantom control. To our knowledge, these studies have only been performed in healthy volunteers. RESULTS: We analyzed the variation in flow measurements made with and without stationary phantom correction in 31 patients with congenital heart disease...

A 20-day-old female child presented with mild respiratory distress since first week of life. On examination there was an ejection systolic murmur at left sternal border. Chest x-ray revealed well-defined mass in the left lung. Echocardiogram showed features suggestive tetrology of fallot with absent pulmonary valve with massively dilated main and left pulmonary artery. We report a rare cause of vascular mass in a neonate due to tetrology fallot with absent pulmonary valve.

Scimitar syndrome is a rare congenital anomaly accounting for about 0.5% of all congenital heart diseases. In its most common form, it is characterized by anomalous drainage of one or all right side pulmonary veins into inferior vena cava or rarely into hepatic, portal, or azygous veins. Other associated anomalies can be hypoplasia or agenesis of right lung, hypoplasia of right pulmonary artery, bronchial anomalies, aberrant blood supply and (or) sequestration of right lung, and variety of intracardiac defects which commonly includes atrial or ventricular septal defects and tetrology of Fallot...

We aimed to ascertain the prevalence of cardiac malformation (CM) and its association with antenatal exposure to an antiepileptic drug (AED) in infants of mothers with epilepsy (IMEs). Women with epilepsy (WWE) are enrolled in Kerala Registry of Epilepsy and Pregnancy (KREP) in the prepregnancy or early pregnancy period and are followed up with a standard protocol until the IMEs are 6 years old. At 3 months postpartum, a cardiologist, blinded to the AED exposure, carried out a clinical examination and echocardiography on all live-born babies...

OBJECTIVE: About 1% of live born children have congenital heart defects (CHD). Knowledge of the true incidence of CHD is important because of the risk of bacterial endocarditis in patients with heart defects. This knowledge could also serve as a basis for research on the etiology of CHD. The aim of our study was to investigate the incidence of CHD in children born in Iceland during a ten year period, from 1990 to 1999. A similar study on CHD was carried out in Iceland for children born 1985-1989...